BACKGROUND Twenty percent of all prostate cancer patients have a positive family history (at least 1 first-degree relative with prostate cancer) and a part of these patients have a genetic predisposition. OBJECTIVE A literature search and analysis of studies investigating incidence, diagnosis, and clinical course of familial compared to sporadic prostate cancer as well as genetic predisposition was performed using PubMed and Embase. RESULTS Risk of prostate cancer depends on number, degree of relationship, and age of onset of affected men in the family. The incidence of familial prostate cancer is higher and the age of diagnosis lower compared to sporadic cases. The clinical course of the disease is comparable, but in individuals with a germline mutation, more intensive therapy is needed due to a more aggressive disease. CONCLUSIONS Crucial for risk assessment is a detailed family history, including creation of a pedigree with cancer family history if necessary. In high-risk families, genetic counselling and annual prostate-specific antigen (PSA) screening beginning at the age of 40 should be performed. Verification of a germline mutation requires more intensive therapy due to more aggressive disease.